Palisaded neutrophilic and granulomatous dermatitis | |
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Specialty | Dermatology |
Palisaded neutrophilic and granulomatous dermaititis (PNGS) is usually associated with a well-defined connective tissue disease, lupus erythematosus or rheumatoid arthritis most commonly, and often presents with eroded or ulcerated symmetrically distributed umbilicated papules or nodules on the elbows. [1] [2]
Typical clinical manifestations include erythematous to violaceous plaques that are symmetrically distributed, skin-colored linear cords involving the lateral trunks, and skin-colored or erythematous papules with crusting, perforation, or umbilication. [3]
Palisaded neutrophilic and granulomatous dermaititis is associated with subacute bacterial endocarditis, [4] ledipasvir/sofosbuvir, [5] allopurinol, [6] Hodgkin’s and non-Hodgkin’s lymphoma, [4] chronic myelomonocytic leukemia, [7] ulcerative colitis, [8] Takayasu arteritis, [4] systemic vasculitis, [8] systemic lupus erythematosus (SLE), [9] sarcoidosis, [10] rheumatoid arthritis, [11] eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome), [8] chronic uveitis, [10] and adult-onset still’s disease. [4]
The underlying illness is the main focus of PNGD treatment. Up to 20% of patients may experience spontaneous resolution of the lesions. Topical corticosteroids, NSAIDs (non-steroidal anti-inflammatory drugs), dapsone, prednisone, colchicine, oral tacrolimus, and TNF inhibitors are among the available treatment options. [3]
Lupus pernio is a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis. The name "lupus pernio" is a misnomer, as microscopically this disease shows granulomatous infiltration and does not have features of either lupus nor pernio.
Granuloma annulare (GA) is a common, sometimes chronic skin condition which presents as reddish bumps on the skin arranged in a circle or ring. It can initially occur at any age, though two-thirds of patients are under 30 years old, and it is seen most often in children and young adults. Females are two times as likely to have it as males.
Desquamative gingivitis is an erythematous (red), desquamatous (shedding) and ulcerated appearance of the gums. It is a descriptive term and can be caused by several different disorders.
Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an autoimmune skin condition on the lupus erythematosus spectrum of illnesses. It presents with red, painful, inflamed and coin-shaped patches of skin with a scaly and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp. The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin. These lesions can last for years without treatment.
Pemphigus erythematosus is simply a localized form of pemphigus foliaceus with features of lupus erythematosus.
Chilblain lupus erythematosus was initially described by Hutchinson in 1888 as an uncommon manifestation of chronic cutaneous lupus erythematosus. Chilblain lupus erythematosus is characterized by a rash that primarily affects acral surfaces that are frequently exposed to cold temperatures, such as the toes, fingers, ears, and nose. The rash is defined by oedematous skin, nodules, and tender plaques with a purple discoloration.
Subacute cutaneous lupus erythematosus is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as poly-cyclic annular lesions or plaques similar to those of plaque psoriasis.
Lupus erythematosus panniculitis presents with subcutaneous nodules that are commonly firm, sharply defined and nontender.
Rheumatoid vasculitis is skin condition that is a typical feature of rheumatoid arthritis, presenting as peripheral vascular lesions that are localized purpura, cutaneous ulceration, and gangrene of the distal parts of the extremities.
Interstitial granulomatous dermatitis with arthritis (IGDA) or Ackerman dermatitis syndrome is a skin condition that most commonly presents with symmetrical round-to-oval red or violet plaques on the flanks, armpits, inner thighs, and lower abdomen.
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:
Neutrophilic dermatosis of the dorsal hands (NDDH) is a skin condition that presents with edematous pustular or ulcerative nodules or plaques localized to the dorsal hands.
Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.
Livedoid vasculopathy(LV) is an uncommon thrombotic dermal vasculopathy that is characterized by excruciating, recurrent ulcers on the lower limbs. Livedo racemosa, a painful ulceration in the distal regions of the lower extremities, is the characteristic clinical appearance. It heals to form porcelain-white, atrophic scars, also known as Atrophie blanche.
Rheumatoid neutrophilic dermatitis, also known as rheumatoid neutrophilic dermatosis, is a cutaneous condition associated with rheumatoid arthritis.
Rheumatoid nodulosis is a cutaneous condition associated with rheumatoid arthritis, characterized by the appearance of multiple nodules, most often on the hands.
Rowell's syndrome was described by Professor Neville Rowell and colleagues in 1963. Patients with the syndrome have lupus erythematosus, annular lesions of the skin like erythema multiforme associated with a characteristic pattern of immunological abnormalities. It is uncommon but occurs worldwide.
Lupus vasculitis is one of the secondary vasculitides that occurs in approximately 50% of patients with systemic lupus erythematosus (SLE).
Cutaneous manifestations of COVID-19 are characteristic signs or symptoms of the Coronavirus disease 2019 that occur in the skin. The American Academy of Dermatology reports that skin lesions such as morbilliform, pernio, urticaria, macular erythema, vesicular purpura, papulosquamous purpura and retiform purpura are seen in people with COVID-19. Pernio-like lesions were more common in mild disease while retiform purpura was seen only in critically ill patients. The major dermatologic patterns identified in individuals with COVID-19 are urticarial rash, confluent erythematous/morbilliform rash, papulovesicular exanthem, chilbain-like acral pattern, livedo reticularis and purpuric "vasculitic" pattern. Chilblains and Multisystem inflammatory syndrome in children are also cutaneous manifestations of COVID-19.